CLASSIFICATION OF PTOSIS

1. Neurogenic
Third nerve palsy Third nerve misdirection Horner syndrome Marcus Gunn jaw-winking syndrome Myasthenia gravis Myotonic dystrophy Ocular myopathies Simple congenital Blepharophimosis syndrome

2. Myogenic

3. Aponeurotic 4. Mechanical

PTOSIS
1. Evaluation
Pseudoptosis True ptosis Neurogenic ptosis Myogenic ptosis Aponeurotic ptosis Mechanical ptosis

2. Classification

3. Treatment options

Causes of pseudoptosis

Lack of lid support

Contralateral lid retraction

Ipsilateral hypotropia

Brow ptosis - excessive eyebrow skin

Dermatochalasis - excessive eyelid skin

Marginal reflex distance

Distance between upper lid margin and light reflex (MRD)

Mild ptosis (2 mm of droop)

Moderate ptosis (3 mm)

Severe ptosis (4 mm or more)

Upper lid excursion

Reflects levator function

Normal (15 mm or more)

Good (12 mm or more)

Fair (5-11 mm)

Poor (4 mm or less)

Vertical fissure height

Distance between upper and lower lid margins Normal upper lid margin rests about 2 mm below upper limbus Normal lower lid margin rests 1 mm above lower limbus Amount of unilateral ptosis is determined by comparison

Upper lid crease

crease

Pretarsal show
fold

Distance between lid margin and lid crease in down-gaze Normals - females 10 mm; males 8 mm Absence in congenital ptosis indicates poor levator function High crease suggests an aponeurotic defect

Distance between lash line and skin fold in primary position of gaze

Bells phenomenon
Upward rotation of globe on lid closure

Good

Poor - risk of postoperative corneal exposure

Left third nerve palsy

Severe unilateral ptosis and defective adduction

Normal abduction

Defective elevation

Defective depression

Right third nerve misdirection

Rare, unilateral Aberrant regeneration following acquired third nerve palsy Pupil is occasionally involved Bizarre movements of upper lid accompany eye movements

Right ptosis in primary position

Worse on right gaze

Normal on left gaze

Horner syndrome

Caused by oculosympathetic palsy

Usually unilateral mild ptosis and miosis

Normal pupillary reactions Slight elevation of lower lid Iris hypochromia if congenital or longstanding Anhydrosis if lesion is below superior cervical ganglion

Important causes of Horner syndrome

Posterior hypothalamus

Central (first order neurone)

Brainstem disease (vascular, demyelination) Spinal cord disease (syringomyelia, tumours)

Pre-ganglionic (second order neurone)

Superior cervical ganglion

Intrathoracic lesions (Pancoast tumour, aneurysm) Neck lesions (glands, trauma)

Post-ganglionic (third order neurone)

Ciliospinal centre of Budge( C8 - T2 )

Internal carotid artery disease Cavernous sinus mass

Marcus Gunn jaw-winking syndrome

Accounts for about 5% of all cases of congenital ptosis Retraction or wink of ptotic lid in conjunction with stimulation of ipsilateral pterygoid muscles

Opening of mouth

Contralateral movement of jaw

Myasthenia Gravis
1. Clinical features

Uncommon, typically affects young women Weakness and fatiguability of voluntary musculature

Three types - ocular, bulbar and generalized

Edrophonium (Camiston) test

2. Investigations

Electromyography to confirm fatigue Antibodies to acetylcholine receptors CT or MRI for presence of thymoma
Medical - anticholinesterases, steroids and azathioprine Thymectomy

3. Treatment options

Ocular myasthenia
Ptosis Diplopia

Insidious, bilateral but asymmetrical Worse with fatigue and in upgaze Ptotic lid may show twitch and hop signs

Intermittent and usually vertical

Edrophonium test
Before injection Positive result

Measure amount of ptosis or diplopia before injection Inject i.v. atropine 0.3 mg

Inject i.v. test dose of edrophonium (0.2 ml-2 mg) Inject remaining (0.8 ml-8 mg) if no hypersensitivity

Myotonic dystrophy
Release of grip difficult Facial weakness and ptosis

Muscle wasting Involvement of tongue and pharyngeal muscles Ophthalmoplegia - uncommon

Hypogonadism Frontal baldness in males Intellectual deterioration Presenile stellate cataracts

Ocular myopathies

Clinical types

Ocular features

Isolated Oculopharyngeal dystrophy Kearns-Sayre syndrome (pigmentary retinopathy)

Ptosis - slowly progressive and symmetrical Ophthalmoplegia - slowly progressive and symmetrical (no diplopia)

Simple congenital ptosis

Developmental dystrophy of levator muscle Occasionally associated with weakness of superior rectus

Unilateral or bilateral ptosis of varying severity

In downgaze ptotic eyelid is slightly higher

Frequent absence of upper lid crease

Usually poor levator function

Blepharophimosis syndrome

Rare congenital disorder Dominant inheritance

Moderate to severe symmetrical ptosis Short horizontal palpebral aperture Telecanthus (lateral displacement of medial canthus) Epicanthus inversus (lower lid fold larger than upper) Lateral inferior ectropion Poorly developed nasal bridge and hypoplasia of superior orbital rims

Aponeurotic ptosis
Weakness of levator aponeurosis Causes - involutional, postoperative and blepharochalasis

Mild

High upper lid crease

Good levator function

Severe

Absent upper lid crease

Deep sulcus

Mechanical ptosis
Causes

Dermatochalasis

Large tumours

Severe lid oedema

Anterior orbital lesions

Fasanella-Servat procedure
Indicated for mild ptosis with good levator function

Excision of upper border of tarsus, lower border of Muller muscle and overlying conjunctiva

..

Levator resection
Indicated for any ptosis provided levator function is at least 5 mm

Shortening of levator complex

Amount determined by levator function and severity of ptosis

Frontalis brow suspension

Main indications Severe ptosis with poor levator function ( 4 mm or less ) Marcus Gunn jaw-winking syndrome

Attachment of tarsus to frontalis muscle with sling